Case: Extrapyramidal disorders.

Case: Extrapyramidal disorders.Presenter:Arwa H. Al-Onayzan.ID: 215007943.Group:1.

The case: A 58-year-old complaining of nagging pain in his right arm, particularly in his shoulder 3 months ago. The pain developed gradually, without any clear reason. He had no loss of strength and no sensory deficits. Test revealed no obvious abnormalities, except for a movement restriction of the shoulder. His wife observed that her husband decreased arm swing in his right arm during walking. He has difficulty with writing and his handwriting has become smaller. She asked if these symptoms suggest her husband may have Parkinsons disease.

The objectives: The definition of Parkinsons disease. The risk factor. Etiology Pathophysiology. Clinical features.

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Extrapyramidal system The extrapyramidal system composed of:

Any defect to this system it will lead to extrapyramidal disorders. 1- Premotor area 6.2- Caudate nucleus.3- Lentiform nucleus.4- Subthalamic nucleus.5- Red nucleus. 6- Substantia nigra.7- Inferior olivary nucleus.8- Reticular formation.

Voulnatry movment are regulated by 2 main tract:#Pyrimidal tract: Composed of Corticospinal tract and corticobulbar tract.#Extrapyrimidal tract: composed of Basal ganglia and cerebellum.

Extrapyramidal system is composed of groups of nucli.Any defect to this pyramidal system will lead to pyramidal disoders The most common example of the extrapyramidal disoders is Parkinsons dieaseas.

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The definition of Parkinsons disease: Parkinson's disease (PD) is a slow and progressive neurodegenerative disease of the extrapyramidal motor system. Dopamine neurons in the substania nigra are primarily affected and degeneration of these neurons causes a disruption in the ability to generate body movement.

http://www.pdf.org/about_pd5

Risk factor of Parkinsons disease: Age and gender:Prevalence sharply increase with age particularly over 57 years.Prevalence higher in men (1.5:1 male to female). Exposure to toxins:Ongoing exposure to herbicides and pesticides slightly increased risk of Parkinson's disease. Heredity:About 15 to 20% of patient have a family history.

Etiology Idiopathic (Parkinsons disease). MPTP (Methyl phenyl-tetrahydro-pydidine).Drugs:Phenothiazines e.g haloperidol, reserpine and metoclopramide. Trauma:Repeated head injury.(Punch drunks syndrome). Viral infection:Encephalitis lethargica.

Toxin that lead to very severe parkinosims.

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The pathophysiology of Parkinsons disease

The Anatomy of basal ganglia The components of basal ganglia: Caudate nucleus. Putamen. Globus pallidus. Substantia nigra. Subthalamic nucleus.

striatumLentiform nucleus.

The function of basal ganglia is two:Motor function: control complex pattern of motor activity like writing.Non motor function: The basal ganglia also play a role in some cognitive processes like learning, emotions, motivationMemory.9

Substantia nigraConsist of two part:Pars Compacta: has dopaminergic neurons serves mainly as input to the basal ganglia circuit, supplying the striatum with dopamine. Pars reticulata: has GABAergic neurons serves mainly as output, conveying signals from basal ganglia to other brain structures.

The pathophysiology Basal ganglia:Control the movement. DopamineInhibitory neurotransmitter releaseBy Nigro-striatal fibers in basal ganglia. AcetylcholineExcitatory neurotransmitter releaseBy intra-striatal fibers in basal ganglia. Without dopamine, inhibitory influences Are lost and excitatory mechanisms Neurons of basal ganglia are over stimulated muscle tone, tremors & rigidity.

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Con.. The pathological hallmark of PD are : The presences of the neural inclusion called Lewy bodies. And loss of the dopaminergic neurons from the pars compacta of the substansia nigra in the midbrain that project to the striatum of the basal ganglia. Lewy body become gradually spread as the condition progress to even to the cerebral cortex. The extent of nigro-striatal dopaminergic cells loss correlate with the degree of akinesia.

lewy bodiesare abnormal aggregates ofproteinthat develop insidenerve cellsinParkinson's disease(PD),12

Clinical feature In most patients, the disease begins insidiously. Initial symptoms: Tiredness Mental slowness. Depression. Aching limbs. Small handwriting (micrographia).

Clinical feature

Tremor A resting tremor of one hand is often first symptoms. The tremor is characterized as: Maximal at rest, lessening during movement and absent during sleep. Increased by emotional tension or fatigue. Often involving the wrist, finger and foot.

The jaw and toungue may effected but not the voice.Tremor may become less prominent as the dieases progress15

Rigidity Develops without tremor in many patients (case). Stiffness on passive limb movement is described as lead pipe. When stiffness occur with tremor, a ratchet like jerkiness is felt this called cogwheel rigidity.

Rigidty = increased tone causes stiffness and flexed posture.lead pipa type of increased muscle tone due to an extrapyramidal lesion in which pathologic resistance to passive extension of a joint is constant throughout the range of motion.

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HypokinesiaHypokinesia mean slowness of voluntary movement and a reduction in autonomic movement.Such as: Swinging the arm while walking. The patients face relatively immobile (mask face). Infrequent blinking, open mouth and drooling. Patient have difficulty in fine and rapidly alternating movement such as writing (micrographia).

Hypokinesia mean slowness of voluntary movment and a reduction in autonomic movmentMask face patient will appear like depressed. Micrographia writing in very small letters.Rigidity and hypokinesia may contribute to muscular ache.17

Abnormal gait & posture patient will find it difficult to get up from bed or chair and tend to adopt a flexed posture on walking. Shuffling gait: The gait is characterized by small, shuffling steps and absence of arm swing. Festinating gait:The patient tend to walk withincreasing the speed to prevent a fall.

Advanced cases (because of the altered center of gravity that result from the abnormal posture.18

Speech & cognition Speech is initially monotonous then progress to dysarthria. Cognitive impairment is 80% common in late stage Dementia may develop in late stages. Visual hallucination and psychosis are not common. Depression is common.

To sum up:

The definition of Parkinsons disease. The risk factor. Etiology Pathophysiology. Clinical features. 20

Q1:What other symptoms could you look for to confirm the diagnosis of Parkinson's disease?Physical Abnormalities in Parkinsons General:Expressionless face.Dysarthria.Flexed posture.Impaired postural reflexes.Tremor:Resting tremors ( start unilateral, Asymmetrical, just at rest).Usually first in fingers.May affect arms, legs, feet, jaw and tongue.Gait:Shortened stride.Rapid, small steps, tendency to run (Festination).Reduced arm swing.Rigidity:Cogwheel type, Mostly UL.Lead pipe type, Mostly legsBradykinesia:Slowness in initiating movements.Impaired fine movement (finger).

Quiz time!!Q1) Give two example of the clinical feature ?? Q2) This phenomenon called??

Any Question?

reference Kumar & Clarks Clinical Medicine (Ninth Edition) by Parveen Kumar and Micheal Clark (P. 852-854). The Merk Manual (Nineteenth Edition) (P. 1765-1771). Guyton and Hall Textbook of Medical Physiology, (12 Edition), (P.693). Parkinson's Disease Foundation website. Short Textbook Of Medical Diagnosis & Management (11 Edition), (P. 660-661).